title: Adrenocortical carcinoma: clinical outcomes and prognosis of 330 patients at a tertiary care center.
abstract: OBJECTIVE
  Adrenocortical carcinoma (ACC) is a rare malignancy with a poor prognosis. Herein, we describe the clinical features and outcomes for a large series of ACC patients.
  
  
  METHODS
  Retrospective review of ACC patients seen at The University of Texas MD Anderson Cancer Center from 1998 through 2011.
  
  
  RESULTS
  A total of 330 patients with median age at diagnosis of 48.5 years; 12 (3.6%) patients were under 18 years. Hormonally functioning tumors represented 41.8% (n=138) of all cases. Surgical resection for the primary tumor was done in 275 (83.3%) patients (45 at MD Anderson (16.4%)). For those who had surgical resection, the median local-recurrence-free time was 1.04 years. Factors associated with local recurrence included positive surgical margins (P=0.007) and advanced disease stage (P=0.026). Median overall survival time for all patients was 3.21 years. Median survival times were 24.1, 6.08, 3.47, and 0.89 years for stages I, II, III, and IV respectively. In multivariable analysis, older age, functioning tumors, and higher disease stage remained significant prognostic factors associated with poor survival.
  
  
  CONCLUSIONS
  ACC prognosis remains poor with the use of currently available treatments. Older age, functioning tumors, and incomplete resections are clinical factors associated with poor survival. Surgical expertise is important to achieve complete resections and to improve outcome.
authors.affiliation: Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center (UTMDACC), Houston, TX.
authors.forename: Montserrat
authors.initials: M
authors.lastname: Ayala-Ramirez
authors.affiliation: Department of Medicine, St. Louis University, St. Louis, MO.
authors.forename: Sina
authors.initials: S
authors.lastname: Jasim
authors.affiliation: Department of Biostatistics, UTMDACC.
authors.forename: Lei
authors.initials: L
authors.lastname: Feng
authors.affiliation: Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center (UTMDACC), Houston, TX.
authors.forename: Shamim
authors.initials: S
authors.lastname: Ejaz
authors.affiliation: Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center (UTMDACC), Houston, TX.
authors.forename: Ferhat
authors.initials: F
authors.lastname: Deniz
authors.affiliation: Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center (UTMDACC), Houston, TX.
authors.forename: Naifa
authors.initials: N
authors.lastname: Busaidy
authors.affiliation: Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center (UTMDACC), Houston, TX.
authors.forename: Steven G
authors.initials: SG
authors.lastname: Waguespack
authors.affiliation: Department of Investigational Cancer Therapy, UTMDACC.
authors.forename: Aung
authors.initials: A
authors.lastname: Naing
authors.affiliation: Department of Pathology, The UTMDACC.
authors.forename: Kanishka
authors.initials: K
authors.lastname: Sircar
authors.affiliation: Department of Urology, UTMDACC.
authors.forename: Christopher G
authors.initials: CG
authors.lastname: Wood
authors.affiliation: Department of Genitourinary Medical Oncology, UTMDACC.
authors.forename: Lance
authors.initials: L
authors.lastname: Pagliaro
authors.affiliation: Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center (UTMDACC), Houston, TX.
authors.forename: Camilo
authors.initials: C
authors.lastname: Jimenez
authors.affiliation: Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center (UTMDACC), Houston, TX.
authors.forename: Rena
authors.initials: R
authors.lastname: Vassilopoulou-Sellin
authors.affiliation: Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center (UTMDACC), Houston, TX.
authors.forename: Mouhammed Amir
authors.initials: MA
authors.lastname: Habra
mesh_terms: D000293:Adolescent; D000306:Adrenal Cortex Neoplasms / Q000378:metabolism / Q000401:mortality* / Q000473:pathology / Q000601:surgery / Q000628:therapy*; D018268:Adrenocortical Carcinoma / Q000378:metabolism / Q000401:mortality* / Q000473:pathology / Q000601:surgery / Q000628:therapy*; D000328:Adult; D000367:Age Factors; D000368:Aged; D000465:Algorithms; D002173:Cancer Care Facilities / Q000706:statistics & numerical data; D019468:Disease Management; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis / Q000401:mortality; D009367:Neoplasm Staging; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis / Q000401:mortality; D011237:Predictive Value of Tests; D011379:Prognosis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D012307:Risk Factors; D062606:Tertiary Care Centers / Q000706:statistics & numerical data; D013781:Texas; D016896:Treatment Outcome